RESUMO
A 64-year-old man was referred to our dermatology clinic with a diagnosis of Muir-Torre syndrome (MTS), he had a history of multiple sebaceous carcinomas and sebaceous adenomas removed over the years. The patient has also had visceral cancer and had undergone a colon resection 17 years before to treat colon cancer and was recently diagnosed with invasive high-grade urothelial carcinoma of the right ureter. In addition, the patient has an extensive family history of cancer; a pedigree was constructed to document this history (Figure 1). Of note is that the patient's mother and father were second cousins. The patient's father was diagnosed with lung cancer at age 57 and died of colon cancer at the age of 72. The patient's mother died of colon cancer at age 74. The patient has three siblings: a sister and two brothers. The sister died of bone cancer at age 42. One brother had a number of cancers including colon, kidney, and skin cancers and died at age 53. His other brother is alive and has a history of colon cancer, kidney cancer, and ureteral cancer. The patient has five children. He has a 40-year-old son who, at the age of 30, was diagnosed with testicular cancer. His daughters are 47, 44, 39, and 34, with no history of malignancy to date. The patient had three maternal aunts, all of whom succumbed to colon cancer, as well as two paternal uncles who died of lung cancer. The patient's maternal grandfather was a smoker and he also died of lung cancer.
Assuntos
Síndrome de Muir-Torre/complicações , Síndrome de Muir-Torre/diagnóstico , Síndromes Neoplásicas Hereditárias/complicações , Síndromes Neoplásicas Hereditárias/diagnóstico , Idoso , Humanos , Masculino , Síndrome de Muir-Torre/patologia , Linhagem , Neoplasias das Glândulas Sebáceas/complicações , Neoplasias das Glândulas Sebáceas/diagnóstico , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/diagnósticoRESUMO
We describe two pediatric cases of febrile ulceronecrotic Mucha-Habermann disease (FUMHD) with response to pentoxifylline and cyclosporine therapies. Based on our review of the literature, we are presenting the first case of FUMHD treated with pentoxifylline and the third case of FUMHD successfully treated with cyclosporine. These agents may be of therapeutic benefit in the treatment of FUMHD, in part by suppressing tumor necrosis factor-alpha, which we believe may mediate the disease process.
